Inferior vena cava, agenesis

Rare.

There are different types:

-        agenesis of the suprarenal-retrohepatic segment: the infrarenal vena cava is normal and communicates with the azygos vein and the superior caval system. Prevalence: 0.3 % of the general population but in 0.6 to 2 % of patients with a cardiovascular anomaly (dextrocardia, pulmonary stenosis, atrioventricular canal, ASD). This anomaly is also seen in case of situs inversus, asplenia or polysplenia, syndromic biliary atresia, hypoplasia or agenesis of the right kidney

-         agenesis of the infrarenal segment (very rare). It is generally isolated and could be acquired (thrombosis?)

-          sequel of thrombosis in the perinatal period.


The obstruction of the inferior vena cava causes uphill venous stasis (varicose veins, congestion of the abdominopelvic and parietal venous circulation) with a risk of thrombosis, and further congestion of the collaterals, specially the retroperitoneal and perimedullary venous circulation.


Clinical signs:

-        venous thrombosis at the level of the lower limbs or the pelvis (very low risk of embolism)

-        hematuria (intravesical varices)

-        sometimes neurological signs in case of congestion of the epidural veins.

-        imagery: dilation of the azygos and hemiazygos veins, dilation (with varicose veins) of the large veins (iliac, intraabdominal and retroperitoneal)


Anesthetic implications:

thromboprophylaxis; increased risk of bleeding in case of abdominal surgery or on the lower limbs; dilation of the perimedullary veins  with a possible increased risk of vascular injury during a neuraxial block.



References :

-         Ramanathan T, Hughues MD, Richardson AJ.
Perinatal inferior vena cava thrombosis and absence of the infrarenal vena cava.
J Vasc Surg 2001; 33: 1097-9.

-        Kamerath J, Morgan WE.
Absent inferior vena cava resuting in exercise-induced epidural venous plexus congestion and lower extremity numbness.
Spine 2010 ; 18 : e921-4

-        Guillot O, Jacquier Y, Slimani H.
Agénésie de la veine cave inférieure.
Ann Fr Med Urgence 2017 ; 7 : 403-4

-        Chew RR, Lim AH, Toh D.
Congenital absence of inferior vena cava : an under recognised cause of unprovoked venous thromboembolism.
QJM 2018 ; 117-8.

-        Spinnato P, Bazzochi A, Albisinni U.
How the absence of infrarenal inferior vena cava can mimic lymphoma.
Pediatr & Neonatol 2019 ; 60 : 110-1

-        Lambert M, Marboeuf P, Midulla M, Trillot N et al  
Inferior vena cava agenesis and deep vein thrombosis: 10 patients and review of the literature.
Vascular Medicine 2010; 15: 451-9.



Updated: April 2019